B. Husberg et M. Schultzberg, PEPTIDERGIC INNERVATION OF THE INTERNAL ANAL-SPHINCTER IN HIRSCHSPRUNGS-DISEASE, Pediatric surgery international, 11(1), 1996, pp. 33-40
The pathophysiology of the impaired sphincter function in Hirschsprung
's disease is still unclear. The peptidergic innervation of the agangl
ionic large intestine is known to be disturbed. The present study anal
yzes the peptidergic innervation of the aganglionic internal anal sphi
ncter (IAS) in comparison with that of the circular layer of ganglioni
c and aganglionic large intestine. Immunoreactivity for the following
substances was analyzed: vasoactive intestinal polypeptide (VIP), subs
tance P (SP), met-enkephalin (ENK), calcitonin gene-related peptide (C
GRP), somatostatin (SOM), and neuropeptide Y (NPY). All patients were
operated upon with Soave's endorectal pull-through technique and a pos
terior partial myectomy of the IAS. For comparison, specimens of resec
ted IAS from adult patients operated upon for rectal cancer as well as
autopsy specimens from a 2-year-old child were analyzed. Differences
in the density of nerve fibers between the ganglionic and aganglionic
large intestine were in accordance with previous studies. In sections
of normoganglionic IAS moderately dense networks of nerve fibers immun
oreactive for NPY, SOM, and VIP were observed. The occurrence of NPY a
nd SOM was somewhat more frequent here compared to the colonic circula
r muscle coat, whereas the opposite was seen for VIP. In aganglionic I
AS abundant nerve fibers immunoreactive for NPY, SOM, and VIP were obs
erved. Only a few SP-, CGRP-, and ENK-immunoreactive fibers were found
in normal and aganglionic IAS. It is concluded that there were modera
te differences in the peptidergic innervation of the aganglionic IAS a
s compared to the normal ganglionic IAS and the circular muscle coat o
f the ganglionic and aganglionic large intestine.